Breathing requires the action of muscles — especially the dome-shaped diaphragm (the primary breathing muscle) located below the lungs and the intercostals between the ribs — to move air in and out of the lungs. Muscles in the abdomen, chest wall and neck also are involved in breathing. ALS can weaken all of these muscles, to the point that air no longer can move in and out of the lungs effectively. In this state of underventilation, or hypoventilation, the blood can’t exchange carbon dioxide (which is exhaled) for oxygen (which is inhaled).

The effects of underventilation can be wideranging and serious. Difficulties related to respiration, including pneumonia, choking, sleep-disordered breathing, shortage of oxygen to the brain and cessation of breathing, often lead to death for people with ALS. But careful monitoring and a choice of interventions can manage respiratory health in order to avoid a dangerous crisis and prolong life, sometimes by many years.

“It’s so much easier to focus on the positives when breathing isn’t a moment-to-moment battle!”

Many ALS specialists recommend getting a breathing test shortly after diagnosis. This gives baseline measures against which later tests of breathing can be compared. As respiration needs more attention, the ALS physician will recommend regular visits to a pulmonologist and respiratory therapist (RT) for therapies to support breathing.

Pulmonary function tests usually involve breathing into a computerized machine through a mouthpiece. One of the most important functions being measured is forced vital capacity (FVC), or how much air a person can expel as fast as possible after taking a deep breath.

It’s important to see a respiratory specialist who works with neuromuscular disorders. Pulmonologists and respiratory therapists usually deal with lung disorders such as asthma or chronic obstructive pulmonary disease; many are unfamiliar with ALS. You need the respiratory team to understand that the breathing problems in ALS aren’t a result of lung disorders, but of muscle weakness. Treatment for problems with the lungs won’t correctly address ALS needs and can be dangerous.

NOTE: Supplemental oxygen, via an oxygen tank, is not a solution to breathing problems in ALS and may even be harmful. Supplemental oxygen is for people with lung diseases. In ALS, what’s needed is help moving air in and out of the lungs.

weakened or softened voice; speaking in short phrases; inability to sing or shout

difficulty coughing and clearing the throat (weakened abdominal and throat muscles also contribute to this)

Any of these symptoms, seen regularly, should be reported to the ALS physician.

Note: People with ALS don’t usually show the kind of heavy labored breathing associated with overexertion.

“It is much more manageable than I ever imagined. The trach care and suctioning takes very little time in your overall day and the vent does all the rest of the work. The only challenge is managing all the supplies, so talk to others who are vented if you decide to go down that route and find a good supply company.”

Simple ways to minimize respiratory problems

Most important is to avoid exposing your loved one to any type of cold, flu or virus. (A weaker respiratory system can’t effectively fight off infections.) This can push weakening lungs to pneumonia and respiratory crisis. If the person’s respiratory system has shown any weakness, avoid close contact with anyone who is sick or who has been exposed to someone with a contagious illness. It’s also important that caregivers protect themselves from cold and flu — and no hugs and kisses with your loved one if you have a cold.

Raise the head of the bed while the person is lying down, either awake or asleep. You may want to purchase or rent an adjustable hospital bed or see if one is available from the MDA loan closet.

Place more fans in the house to keep air circulating and fresh.

Have the person with ALS and primary caregivers get a flu shot and a pneumonia vaccination.

Increase the intake of fluids, as long as the person doesn’t have trouble swallowing them (see Chapter 5[14]).

Help your loved one perform respiratory exercises, only if prescribed by a doctor or respiratory therapist.

Include respiratory equipment or supplies as part of your preparation for emergencies (see Emergency Preparation[15]).

Ask about medications such as expectorants, which help people cough up respiratory secretions more easily. In some cases, a doctor may recommend bronchodilators, which open up the bronchial tree.

Review with the pulmonary care team a list of medications being taken. Some drugs can have an adverse effect on respiratory function, and can be switched to others with fewer respiratory side effects.

Consider inserting a feeding tube to reduce the danger of aspirating food into the lungs (see Feeding Tubes[16]).

Coughing

“When my husband was vented, the RRT recommended that I purchase a video cart on rollers. It works great. It holds his vent, CoughAssist, humidifier, suction and has another shelf with four plastic drawers that hold supplies.”

Weakened abdominal and throat muscles in ALS diminish the ability to cough.

When a person with ALS can’t cough up mucus and inhaled particles, these can fall back into the lungs, where they can cause irritation and infection. Weakened swallowing muscles also make aspirating (inhaling) food and liquids into the lungs more likely, which can irritate the lungs and bronchial tree. This can lead to pneumonia or acute choking. (See Choking[17].)

If your loved one’s ability to cough isn’t sufficient to clear secretions from the throat, lungs and trachea, you can learn manual or mechanical assisted coughing techniques. When coughing is weakened, it’s important for caregivers to provide daily help in keeping lungs clear.

The simplest method is to place your hands or arms over the person’s stomach, just below the ribs and under the diaphragm, while he or she is lying down or sitting. After they take one or two deep breaths, firmly push in and upward toward the ribs as they cough. This will force air from the lungs more quickly and help push any secretions out through the mouth. Have a tissue or portable suction device available to collect any mucus coughed up.

This is best done on an empty stomach. If the person with ALS gets nauseous or throws up during the process, try gentler pushing. An increase or other change in mucus may indicate a respiratory infection needing medical attention.

NOTE: Let a respiratory therapist demonstrate these techniques so you’ll be sure to perform them correctly. If the person with ALS has any discomfort or bad reaction, consult the therapist or physician.

For mechanical assistance, the CoughAssist from Respironics[19], also called an insufflator-exsufflator, is a very handy, effective device. A mask is put over the mouth, and the machine blows air into the lungs, then reverses the flow, simulating a cough.

After either a manual or mechanical assisted cough, you may need to use a portable suction device to remove secretions from the mouth through a small tube. You can rent or buy a suction device (similar to those dentists use) from a medical equipment vendor or borrow one from the MDA equipment program.

Doctors also may suggest these cough assist methods:

expectorants — prescription medications that thin secretions, making it easier to cough and clear the secretions

breath stacking — closing the throat after each breath taken in through a mouthpiece and then coughing

oscillation vests or airway clearance systems to “shake up” mucus in the chest

Progressive breathing difficulties are part of the natural history of ALS. It’s wise to plan ahead, talking with the doctor and each other about dealing with breathing problems as they arise.

A breathing crisis can arise very quickly. Call 911 immediately if your loved one is showing severe signs of underventilation such as struggling for breath or inability to breathe. (See Symptoms of Chronic Underventilation[20].)

A bag valve mask (BVM or Ambu bag) is a normal part of an emergency crew’s resuscitation kit or a hospital crash cart to help someone who’s not breathing. (This is what doctors on TV dramas are calling for when they say, “Bag him!”) Ambu bags serve essentially the same purpose as mouth-to-mouth resuscitation and are available online or through medical supply stores.

“The effects of the vent on my health — oxygen levels that prevent headaches and give me energy, stamina, appetite, and pink cheeks — are equaled by improvements in my quality of life. My days are no longer dominated by shortness of breath, tiredness, and fear of the next episode of lung congestion. My world has re-expanded along with my lungs.”

Emergency medical personnel probably will want to administer oxygen in response to signs of respiratory distress. This is fine only if combined with breathing support. A written explanation from your physician should help explain the situation to emergency personnel. The ALS health care team should be notified of the emergency, and the specialist’s contact information offered to the emergency team.

Planning ahead for assisted ventilation will help you avoid having to make decisions in an atmosphere of panic. (See Discussing options together[21] below.)

Short of full-blown respiratory crisis, other signs, such as continued shortness of breath, fever, malaise or low test readings, require immediate medical attention. These could mean an acute infection.

When FVC reaches a certain low level, usually near 50 percent, the respiratory team will introduce the idea of assisted ventilation. There are two primary types: noninvasive, in which air is delivered through a mask and no surgery is involved; and invasive, in which a tube is surgically inserted in the trachea and attached to a respirator. Assisted ventilation can help your loved one feel less fatigued and safer, by improving sleep and decreasing the potential for respiratory crises. It can rest the breathing muscles and increase energy, allowing for more activities and enjoyment of life.

Discussing options together

Deciding which, if any, assisted ventilation to use in ALS is one of the most important and most personal choices you and your loved one will have to make. Discuss this well in advance of time to act on the decision, and review all the factors involved. An emergency room is a poor environment for making such a critical decision — and a respiratory crisis may decrease your options.

It’s difficult to face these decisions when you’re trying to focus on ways to maintain life as you know it and to hold onto hope for a long survival or research progress. An absolute decision doesn’t have to be made in the first few months after diagnosis. But getting the issues out on the table and exploring the options is important. Keep the door open for a while. The options may look different as time passes.

First, do some research through sources like those suggested in the Resources sections. Talk with your health care team; speak with other families of people with ALS. Check your insurance policy, and see what coverage is offered for respiratory equipment, supplies and day-to-day care.

Don’t expect a consensus of opinion from everyone you consult. Even physicians who are knowledgeable in this area have different opinions, sometimes depending on their religious or ethical values. No one can tell you what’s best for your family.

It isn’t unusual for those in the early stages of ALS to declare they don’t want to live with mechanical interventions such as a feeding tube, wheelchair or tracheostomy. But after living with ALS for some time, and making small adjustments as abilities decline, each of these devices often seems more acceptable. Those with young children or other joys in living, or with certain religious beliefs, may feel that life is worth sticking around for, even if they need machines to help them move, eat and breathe.

Others may choose to decline invasive ventilation, and opt for pain control as their breathing further weakens and stops.

Obviously, this decision is very much an individual one. It should have input from close family members, and be made on the basis of complete information and a time of reflection. Ultimately the choice is made by the person with ALS; his or her wishes can be expressed in a medical power of attorney[23] or living will document.

Most people with ALS who need breathing help begin with noninvasive assisted ventilation (NIV), and some people are able to continue with this approach for many years. At first, the machine only may be needed during sleep — restoring a good night’s sleep for both of you! Later it may need to be available during some daytime hours.

The most common form of noninvasive ventilation is the bilevel positive pressure type, in which air is supplied through a facial mask (interface) on a timed cycle. This involves a small, portable machine that can be placed at bedside. The well-known BiPAP (bilevel positive airway pressure) machine by Respironics delivers air at two pressures — a higher pressure to help the person breathe in more, and a lower pressure so they can breathe out with little resistance.

There are other NIV choices, which the pulmonologist will explain in recommending the best one for the individual situation.

Interfaces

The interface is the mask or other device placed over the nose, mouth, or mouth and nose, so a person can use assisted ventilation.

Ideally the person with ALS should try several interface options to get used to the feeling and practice breathing through a mask. The RT should ask questions about sleep habits, nasal congestion and feelings of claustrophobia, and determine which mask might be most comfortable and supportive.

With patience, the user should get used to a comfortable interface in a few weeks or months. Begin by using it a few minutes at a time, gradually increasing duration.

A face mask is most common, but if users experience panic, claustrophobia or general discomfort at having something over the face, nasal pillows may be preferable. These fit inside the nostrils and put no weight on the face; some users can’t even tell they have them on.

Some people use nasal pillows in the daytime and a face mask at night. Nasal pillows also make it easier to turn the head and wear eyeglasses.

Straps around the head hold the mask in place. If air escapes through the mouth at night, chin straps can keep the mouth closed.

Masks are designed to seal around the nose and/or mouth. When they leak, they can blow air into the face and eyes, and decrease the effectiveness of ventilation. Try different masks until finding one that seals properly. Gel- and air-cushioned masks are becoming more common, are lighter on the face and seal better. Those with bulbar symptoms may have the most trouble keeping the mask sealed. Some find the best solution is a custom-fitted mask or nasal plug made through the hospital’s respiratory therapy department.

Some masks irritate the skin on the bridge of the nose or around the mouth. One solution is tucking moleskin, cotton or soft felt just under the nose part, being careful not to break the seal. Carefully clean and dress any sores or wounds. Apply an antibiotic ointment such as Neosporin while the respirator isn’t in use.

All parts of an NIV device require regular cleaning and sterilization, including the tubing, filters and mask. Filters need regular replacement, and an enzyme cleaner may be required for the mask.

Settings

For NIV to be as comfortable and effective as possible, settings for air volume and pressure have to be correct and supportive. If they aren’t, breathing distress will continue. Users have complained that their cheeks are puffed out or air bloats the stomach. These discomforts result from an air pressure or volume that’s too high.

Usually your respiratory team, including representatives of the device manufacturer, will set the pressure levels based on a sleep study, or begin at low levels and adjust them with frequent monitoring. They can be increased gradually to the level your pulmonologist considers most supportive and your loved one can tolerate.

Setting up the equipment, interfaces and follow-up requires experience and careful adjustment to make it work properly for each person, although some machines come with software or controls and information that helps adjust them correctly. The respiratory team or the vendor supplying the equipment should be available to make adjustments as needed, or to perform overnight oximetry at your home. Try to find a support person with knowledge of ALS or neuromuscular diseases.

“I really wish [my husband] would have let me check more into how people can live with a vent and stay home with their family. He said he was never going to live that way. I sure wish I had pushed it more and showed him how others were living. Then just maybe this year he would have been here to see his son get his high school diploma, start college and see how well his other son enjoys singing music. How very proud he would have been of them.”

Oximetry is a measure of oxygen saturation that tells whether the respiratory device is providing enough support. A pulse oximeter should be used regularly to check oxygen saturation while using NIV. An RT can check the pulse oximetry on visits, or you can buy one for about $350-$400.

NOTE: Respiratory function is too important for guesswork. It’s OK to make small NIV adjustments at home, but don’t hesitate to call experts whenever you have concerns about the equipment, no matter how many visits it takes to get it right.

Several studies have suggested that NIV can actually slow the decline in respiratory function and make a significant difference in survival time in people with ALS. This applies to those with moderate or no weakness of the mouth and throat muscles. Those with more severe bulbar (mouth and throat) muscle involvement may be less likely to tolerate NIV and may have to consider invasive ventilation sooner in the disease course.

People with ALS and physicians hold strong opinions about the long-term use of NIV — for and against. Whether your loved one decides to use NIV or invasive ventilation, close monitoring by respiratory experts is vital.

Facial, mouth and throat weakness can reduce necessary jaw closure and ability to use a mouthpiece with NIV.

Bulbar muscles are weakened so that the person can’t speak or swallow or keep saliva out of the airway.

The hours the person needs to use NIV increase from overnight to most of the day. Going out becomes more difficult if the person resists wearing the mask in public.

Because pressure-based NIV can only assist breathing, as respiratory capacity deteriorates, the user slides back into the fatigue, poor appetite and anxiety of pre- NIV days.

Pneumonia or a simple chest cold result in a respiratory crisis. As lungs become congested, NIV reaches its pressure limit more quickly, and less air is delivered.

The person with ALS has problems swallowing and often aspirates food or saliva into the lungs, creating more respiratory distress.

Invasive ventilation can allow the longest possible survival with ALS.

The most permanent type of ventilation is the positive-pressure ventilator with a surgically created tracheostomy. A ventilator is attached by a breathing hose to the tracheostomy tube, delivering air through the neck into the trachea (windpipe) on a timed cycle.

Tracheostomy surgery is considered minor and often can be done under local anesthesia with sedation. It’s usually followed by several days or weeks of rehabilitation during which caregivers can learn how to clean and maintain the tracheostomy tube, change supplies and perform suctioning. Ask nurses and respiratory therapists all the questions you can think of during this phase.

Quality of life

At one time, people using invasive ventilation were kept in institutions because of their machinery. Nowadays, many people on total ventilatory support continue working, traveling, socializing and enjoying life. As with many of the changes brought by ALS, once the difficult decisions are made, the adjustment may prove relatively easy and well worth the effort. With the face now free of the NIV interface, some people are far more sociable.

Today’s vents are small, portable, relatively quiet and easily can be carried on a wheelchair. For those who can still speak, a speaking valve often can be added to the inflatable cuff at the end of the tracheostomy tube; this allows air to travel to the vocal cords, enabling speech. (If it’s necessary for the cuff to be inflated all the time, the person won’t be able to speak.)

In addition, a tracheostomy can provide a great feeling of safety. Permanent vents have alarms to alert caregivers to congestion or a disconnected tube. And invasive ventilation can return some energy as it relieves the exhaustion of poor sleep, prolonged coughing and labored breathing.

Problem solving

However, maintenance of invasive ventilation does require greater effort by the caregiver. Problems that arise may not be easy to fix without help.

Whenever the trach or vent tubing is moved, it sets off an aggravating but not painful coughing spell. Vent hoses may pull on the tracheal opening, causing a sore throat-type ache. These problems are usually relieved by repositioning the hoses; a caregiver may require help from an RT or equipment provider to make this adjustment.

Discomfort of the skin around the trach indicates irritation or infection and usually is easily treated with ointment.

The most annoying discomfort is the same as with an NIV mask — air leaks. As with a mask, adjusting the hose position may help, but persistent problems may require a medical procedure to increase the size of the trach.

Trachs carry a risk of incision infection, and increased respiratory infections are reported. At the same time, the trach is easier than NIV for clearing lung secretions and controlling respiratory status.

Some people are prone to granulation tissue, an excessive growth of new tissue stimulated by the trach incision and presence of the trach tube. This tissue is delicate and bleeds easily and may make trach changes difficult. Granulation tissue can be handled with cortisone, silver nitrate and, in some cases, laser removal. Seek medical advice.

Trach users need a backup ventilator.

The battery operating the vent has time limits and will need to be charged regularly. An extra battery is necessary.

Vents often have temperature limitations, so avoid excessive cold or heat.

When the ventilator is installed, contact the local electrical and/or gas power company and explain that this is a device required for life. Request a form to protect against the power being turned off and have it signed by the ALS doctor. A generator or other backup power strategies are advisable.

In 2011, the U.S. Food and Drug Administration (FDA) approved as a "humanitarian use device" a new method of respiratory assistance for people with ALS-caused breathing difficulties but with adequate preservation of the diaphragm muscle and the phrenic nerves, which stimulate it.

A humanitarian use device is one that the FDA has determined does not pose an unreasonable risk of illness or injury and for which the probable benefit to health outweighs the risk of illness or injury from its use.

Diaphragm stimulation, or pacing, involves a rhythmic stimulation of the major breathing muscle through electrodes, which must be surgically implanted into the diaphragm. The electrodes send signals to the diaphragm muscle, causing it to contract and assist breathing efforts.

The NeuRx Diaphragm Pacing System (DPS), by Synapse Biomedical, can be used in conjunction with noninvasive ventilation (e.g., BiPAP) or by itself. The DPS doesn’t slow or stop the progression of ALS, but it may delay the need for a tracheostomy, and may improve sleep and quality of life. MDA is supporting further studies of the device's effectiveness in ALS.

Those caring for people using invasive ventilation move into a new level of caregiving. A person with a trach must have someone available at all times to suction saliva and mucus plugs and respond to emergencies, such as a detached tube or power failure. However, nurses aren’t required. Anyone, even an older child, can be taught how to suction and handle vent alarms.

Caregivers must decide who should be given the responsibility of ventilator care. For those hiring outside help, be aware that many home health care agencies only will provide registered nurses for people with trachs (see Chapter 8[38]). This introduces a cost of several thousand dollars a month.

In addition, new supplies are required, including dressings, tubing, filters and batteries. See Chapter 7[39] to learn which costs may be covered by Medicare or private insurance.

It’s possible for a person with a trach to shower but extreme care must be taken not to get water into the opening. Use a cloth or plastic wrap to keep water out.

Daily care for people with invasive ventilation includes:

Cleaning around the trach, as part of bathing or washing up, using ordinary soap and clean washcloths. If there’s a lot of mucus drainage around the tube, recurring infections or redness, more cleaning or use of ointments may be required.

Dressings where the tube connects to the body must be changed.

The inner cannula, a tube within the trach tube, must be cleaned daily, usually with dish soap or hydrogen peroxide. Some health care providers suggest changing the inner cannula every day.

Trach ties, which hold the device and dressings around the neck, should be changed when they’re damp or dirty.

The outer trach tube needs to be changed regularly — some experts advise once a day, some say once a week. People prone to granulation tissue need to have the tube changed more frequently, as do those with respiratory infections. Changing the tube is usually a simple procedure.

Suctioning is necessary to remove mucus or saliva that the person with ALS can’t cough up. Some days people need suctioning two or three times, other days they require it a dozen or more times.

Generally suctioning can be done by a caregiver with a nonsterile disposable glove (not reused) and catheter (changed daily or several times a day). The respiratory care team will provide instructions. The caregiver passes a catheter attached to a suction device through the trach tube and down into the airway. This must be performed carefully because suctioning itself can push bacteria from the upper airway down into the deeper and normally sterile lung passages, adding to the risk of respiratory infections, including serious pneumonias. Be sure the gloved hand doesn’t touch anything but the catheter.

You also can use an insufflator-exsufflator such as Respironics’ CoughAssist (see Assisted Coughing[40]), to fit onto the trach tube and pull out secretions from both lungs at once. Use of the CoughAssist can reduce, if not eliminate, the need for suctioning.

In a person with ALS who’s using full-time invasive ventilation, muscles will continue to weaken to virtually total paralysis. If no alternative communication strategy has been learned, the person will be “locked in” with no way to move or communicate.

In this situation, or for other personal reasons, people with ALS sometimes wish to detach the ventilator and let nature take its course. It’s important to discuss his possibility with the loved one so that his or her wishes are known before it becomes impossible to communicate. (See Advanced Directives[43].)

Ask the doctor about the options and consequences, and after a decision is made, be sure the doctor is aware of it. Putting choices in writing is a greater guarantee that your loved one’s wishes will be followed; don’t assume the health care team knows these wishes without being told.

Doctors can make the individuals with ALS completely comfortable and remove the ventilator. Medication is given to ease pain and respiratory distress.